Odontomes & Odontogenic Tumors II

Classification of Odontogenic Tumors

Benign Odontogenic Tumors
Epithelial lesions:
1) Without odontogenic mesenchyme Ameloblastoma Sequamous odontogenic tumor* Calcifying epithelial odontogenic tumor Adenomatoid odontogenic tumor Keratinizing cystic odontogenic tumor
(

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Classification of Odontogenic Tumors

Benign Odontogenic Tumors
Epithelial lesions:
(2) With odontogenic mesenchyme (mixed) Ameloblastic fibroma and fibrodentinoma Ameloblastic fibro-odontoma Odontoamaloblastoma Calcifying odontogenic cyst and dentinogenic ghost cell tumor Calcifying cystic odontogenic tumor (calcifying odontogenic cyst) and dentinogenic ghost cell tumor Complex odontome Compound odontome

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Classification of Odontogenic Tumors

Benign Odontogenic Tumors

Mesenchymal lesions:
Odontogenic fibroma Myxoma Cementoblastoma

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Classification of Odontogenic Tumors

Malignant Odontogenic Tumors Odontogenic carcinomas:
Malignant ameloblastoma/ameloblastic carcinoma Primary intra-osseous carcinoma Clear cell odontogenic carcinoma Malignant variants of other epithelial tumors Malignant change in odontogenic cysts

Odontogenic sarcomas:
Ameloblastic fibrosarcoma Ameloblastic fibro-odontosarcoma
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Classification of Odontogenic Tumors

Tumors of Debatable Origin Melanotic neuroectodermal tumor of infancy Congenital gingival granular cell tumor (congenital epulis)

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Ameloblastic Fibroma
Presents in young age groups. Rare benign tumor with both epithelial and mesenchymal components neoplastic. Slowly enlarging swelling mostly in posterior parts of mandible. Important to differentiate from ameloblastoma since it is not invasive and does not require aggressive therapy. Recurrence rates of ~18%.

Ameloblastic Fibroma: Radiographic Features

Radiographically, well-defined unilocular radiolucency. May be associated with an unerupted tooth and mimic a dentigerous cyst.

Ameloblastic Fibroma: Histopathologic Features

Proliferating strands of odontogenic epithelium lying in highly cellular fibroblastic tissue resembling dental papilla. Recall In ameloblastoma: the stroma rarely contains fibroblast (not highly cellular) stellate cells are much more abundant Follicles may be larger

Ameloblastic Fibroma: Histopathologic Features

If dentin is present, the tumor is designated ameloblastic fibrodentinoma.

If there is also enamel formation, they are designated ameloblastic fibroodontoma.

Calcifying Cystic Odontogenic Tumor (Gorlin Cyst) & Dentinogenic Ghost Cell Tumor
The calcifying cystic odontogenic tumor previously known as Calcifying Odontogenic Cyst (COC) A grossly cystic tumor and may be a hamartoma rather than a true neoplasm. The dentinogenic ghost cell tumor is histologically similar except that it is solid, i.e. it doesnt show cystic component However, there is increasing evidence that they are distinct entities. Both present centrally within the jaws, but peripheral gingival lesions also occur.

Calcifying Cystic Odontogenic Tumor (Calcifying Odontogenic Cyst or Gorlin Cyst)

Clinical Features
The calcifying cystic odontogenic tumor usually occurs below age 40.

75% intraosseous.
Majority arise anterior to 1st molar in either jaw. Slowly enlarging swelling.

Calcifying Cystic Odontogenic Tumor (Calcifying Odontogenic Cyst or Gorlin Cyst)

Radiographic Features
Well-defined unilocular or multilocular raadiolucency with variable amounts of radiopaque material.
May be associated with crown of an unerupted tooth. Usually responds to conservative treatment.

Calcifying Cystic Odontogenic Tumor (Calcifying Odontogenic Cyst or Gorlin Cyst)

Histopathologic Features
Cyst lined with epithelium similar to ameloblastoma, basal columnar cells with nuclei polarized away from the underlying connective tissue. Stellate reticulum-like cells with keratinization in the center. A characteristic feature is the presence of ghost cell which are odontogenic cells for which only the original outline can be seen, these cells may undergo calcification There may be enamel or dentinlike masses.

Calcifying Cystic Odontogenic Tumor (Calcifying Odontogenic Cyst or Gorlin Cyst)

Histopathologic Features

Dentinogenic Ghost Cell Tumor

The dentinogenic ghost cell tumor tends to occur in an older age group than the calcifying cystic odontogenic tumor. It may deposit some dentin Some cases respond to conservative treatment, other cases pursue a more aggressive course and are locally invasive like ameloblastoma.

Odontogenic Fibroma and Myxoma

Derived from mesenchymal dental tissues. May arise in relation to root, crown, or replace a tooth. These different presentations reflect the different mesenchymal tissues that may give rise to the tumors (PDL, dental follicle, and dental papilla).

Odontogenic Fibroma
Clinical Features
Peripheral odontogenic fibroma arises on the gingiva as a fibrous epulis. The central variant is an uncommon, welldemarcated benign fibroblastic neoplasm.

Readily enucleated with no tendency for recurrence.

Odontogenic Fibroma
Histopathological Features
Cellular fibro-collagenous tissue containing inactive odontogenic epithelial islands.
Foci of cementum-like and dentin-like material may be present.

Odontogenic Fibroma
Histopathological Features

Odontogenic Myxoma
Clinical Features
More common than odontogenic fibroma. Myxoma is benign but locally invasive.

Odontogenic Myxoma
Radiographic Features
Multilocualr radiolucency, soapbubble, or tennisracket appearance, often with welldefined margins. Root resorption.

Odontogenic Myxoma
Histopathological Features
Nonencapsulated, infiltrative pattern of growth. Stellate, fibroblast-like cells with long anastomosing processes. Abundant connective tissue ground substances (gelatinous or mucoid material) predominnaltly glycosaminoglycans.

Odontogenic Myxoma
Histopathological Features
Some cases contain a few strands of odontogenic epithelium. Variable amounts of collagen, and if prominent it may be designated as myxofibroma or fibromyxoma.

Odontogenic Myxoma: Behavior

Unlike fibroma, the locally invasive growth of myxoma and friable myxoid tissue predispose to local recurrence. Recurrence rates of 10-30%.

Cementoblastoma
Although cementum is considered to be a modified form of bone, cementoblastoma is still classified as an odontogenic tumor because of its unique association with the root of a tooth. Identical to osteoblastoma except for association with tooth roots.

Cementoblastoma
Clinical Features
A rare, benign neoplasm. Mostly seen < 25 years of age. Usually mandibular molarpremolar area attached to a root of a tooth. Most cases involve mandibular 1st permanent molar. Slowly enlarging swelling which sometimes gives rise to pain. Involved tooth is vital. May recur if incompletely removed.

Cementoblastoma
Radiographic Features
well-circumscribed, mottled or dense radiopaque mass with a radiolucent margin attached to the root of a tooth which usually shows resorption.

Cementoblastoma
Histopathologic Features
Mass of calcified cementum-like tissue containing scattered cells lying in lacunae growing in continuity with the apical cemental layer of the root.

Cementoblastoma
Histopathologic Features
Around the periphery and in actively growing parts, extensive sheets of uncalcified matrix formed by plump, deeply staining cementoblasts.

Malignant Odontogenic Tumors

1. Malignant Ameloblastoma & Ameloblastic Carcinoma
Malignant ameloblastoma: Very rarely, an ameloblastoma shows true malignant behavior with metastasis. The primary and metastatic neoplasms show histopathologic features of typical ameloblastoma, i.e. no atypia is seen. If only lung metastasis is seen the possibility of seeding due to aspiration during surgery should be considered. If the tumor shows features of atypia, it is given the diagnosis of ameloblastic carcinoma.

Malignant Odontogenic Tumors

2. Malignant Change in Odontogenic Cysts
Cystic degeneration may occur within an existing carcinoma . Likewise carcinoma may arise within an adjacent cyst. Dysplasia is seen sometimes in long standing odontogenic cysts.

A squmaous cell carcinma may present with clinical and radiographic or histological evidence consistent with an origin from an odontogenic cysts. The cysts include radicular, residual, dentigerous and keratocysts.

Malignant Odontogenic Tumors 3. Primary Intraosseous Carcinoma

Rarely, carcinomas arise within the jaws without a preexisting cyst. They are presumed to arise from residues of odontogenic epithelium. They may also be referred to as odontogenic carcinomas.

Malignant Odontogenic Tumors 4. Odontogenic Sarcomas

Malignant counterpart of ameloblastic fibroma and related lesions. Fibrosarcomas containing non-neoplastic odontogenic epithelium and occasionally dental hard tissues, e.g. ameloblastic fibrosarcoma, fibrodentinosarcoma.

Tumors of Debatable Origin

Melanotic Neuroectodermal Tumor of Infancy
Rare tumor which occurs in infants usually in the first year of life.
Most common in maxilla Symptomless mass expanding the bone.

Tumors of Debatable Origin

Melanotic Neuroectodermal Tumor of Infancy

Evidence of neural crest origin. Most are benign and recurrence is uncommon following conservative excision.

Tumors of Debatable Origin

Congenital Epulis
Also known as: congenital gingival granular cell tumor, congenital granular cell epulis, congenital epulis of the newborn. A unique and rare congenital tumor of the alveolar mucosa of the jaws that occurs only in newborns. 10 times more common in females. Predilection for anterior maxilla.

Tumors of Debatable Origin

Congenital Epulis
Large, closely packed granular cells. Resembles granular cell tumor, but histogenesis unknown. Benign , probably reactive lesion! Doesnt recur after excision